Multiple symmetric lipomatosis, also called Madelung's syndrome, is a rare disease charac terized by the accumulation of rapidly growing benign fat tissue, particularly in the cervical region, back, and more rarely in the extremities and face. It is generally associated with chronic alcoholism. Surgical treatment is palliative, and relapse is common. This paper reports the case of a patient with multiple symmetric lipomatosis followed by a review of the literature addressing the diagnosis and treatment of this condition.Keywords: Multiple symmetrical lipomatosis. Lipomatosis. Adipose tissue/pathology. RESUMOA lipomatose simétrica múltipla ou síndrome de Madelung é uma doença rara, caracterizada por depósitos de tecido adiposo de caráter benigno, de rápido crescimento, principalmente em região cervical, dorso e, mais raramente, membros e face, em geral relacionados ao al coolismo crônico. O tratamento cirúrgico é paliativo e a recidiva é comum. Neste trabalho é relatado um caso de paciente portador de lipomatose simétrica múltipla, seguido de revisão de literatura enfocando diagnóstico e tratamento dessa afecção.Descritores: Lipomatose simétrica múltipla. Lipomatose. Tecido adiposo/patologia.
Background and objective Rheumatoid arthritis (RA) is a rheumatic disease mainly affecting women. Prolactin (PRL) is a sex hormone, which apart from inducing lactation, has also immunomodulatory properties. High prolactin levels are associated with an increased disease activity postpartum and some studies have shown that bromocriptin, decreasing prolactin levels, improves clinical activity of patients with RA. Furthermore, hyperprolactinemia is observed in 6% of RA patients compared to 3% in the normal population. Recently, the prolactin receptor (PRLR), belonging to the family of cytokine receptors, has been described in atherosclerotic plaques, mainly on macrophages. The objective of the study is to determine (1) the level of PRL in RA patients related to treatment effect (2) PRLR expression in synovial tissue of RA, psoriatic arthritis (PsA) and osteoarthritis (OA) patients (3) the phenotype of the PRLR expressing cell. Material and methods Serum prolactin levels were measured using immunofl uorescent metric assay in patients with RA before and after tumour cecrosis factor α (TNFα) blockade (n=118). The expression of PRLR was determined in synovial tissue (ST), of RA (n=91), PsA (n=15) and OA (n=9) patients. Immunofl uorescence (IF) was used to detect the PRLR expressing cell type. Results Hyperprolactinemia was observed in 4,2% of the patients with RA (PRL level 16-36 μg/l). Prolactin level is respectively highest in premenopausal, postmenopausal females compared to male. The level of prolactin was decreased in the group of responders compared to the non-responders to TNF treatment, respectively 7.0 (2.0-36) and 8.5 (4.0-19) (median (range) μg/l; p=0.048). Higher tertiles of prolactin levels, still within physiological range, were associated with reumafactor positivity (p=0.031), anticyclic citrullinated peptide (p=0.075) and erosive disease (p=0.095). The number of patients expressing PRLR in the synovium was comparable between RA and PsA (66% and 73%, respectively) versus 25% of the patients with OA (p=0.050). The levels of PRLR expressions were signifi cantly higher in RA and PsA compared to OA, 0.055 (0.000-5.673), 0.182 (0.000-5.336) and 0.000 (0.000-0.908) (median (range) IOD/nuclei per mm 2 ; p=0.024), see fi gure 1. There was no signifi cant difference in PRLR expression between males and (pre/postmenopausal) females. Using IF, colocalisation was observed with markers of macrophages and endothelial wall. Conclusion Higher levels of prolactin were found in patients not responding to anti-TNF treatment. The expression of the prolactinreceptor in synovial tissue, mainly by macrophages, is higher in the infl ammatory diseases (RA and PsA) compared to OA. Our data suggest a role of prolactin as sexhormone in rheumatoid arthritis.
INTRODUÇÃO: O tumor sólido pseudopapilar do pâncreas, descrito pela primeira vez por Frantz em 1959, corresponde a uma neoplasia rara dos tumores pancreáticos de origem não endócrina. OBJETIVO: Discutir um caso atípico de neoplasia abdominal, com diagnóstico de tumor sólido pseudopapilar do pâncreas. DISCUSSÃO DE CASO: E.C.M.A., 19 anos, sexo feminino, queixando-se de dor epigástrica há 7 meses, associada a náuseas e vômitos frequentes. A ultrassonografia revelou a presença de uma massa sólida em região epigástrica, corroborando com a tomografia computadorizada, onde se evidenciou uma volumosa lesão expansiva epigástrica, predominantemente hipodensa, de limites bem definidos. Foi submetida à laparotomia com pancreatectomia de corpo e cauda. Apresentou boa evolução no pós-operatório, obtendo alta no 5º dia. CONSIDERAÇÕES FINAIS: A ressecção completa oferece aos pacientes bom prognóstico, com taxas de sobrevida de 90%.
Background Rheumatoid arthritis (RA) mainly affects women. Prolactin (PRL) is a sex hormone with immunomodulatory properties. High prolactin levels are associated with increased disease activity postpartum, and that the PRL-inhibitor bromocriptine improves disease activity of patients with RA. Hyperprolactinemia is observed in 6% of RA-patients, compared to 3% of healthy individuals. The prolactin receptor (PRLR), belonging to the family of cytokine receptors, has been described in atherosclerotic plaques, mainly on macrophages. Objectives The objective of the study is to determine 1) the level of PRL in RA-patients related to treatment effect 2) PRLR expression in synovial tissue of RA, psoriatic arthritis (PsA) and osteoarthritis (OA) patients 3) the phenotype of the PRLR expressing cell. Methods Serum prolactin levels were measured using immunofluorescent metric assay in patients with RA before TNF-α blockade (n=98). The expression of PRLR was determined in synovial tissue (ST) of RA (n=91), PsA (n=15) and OA (n=9) patients using digital image analysis. Immunofluorescence (IF) was used to detect the PRLR expressing cell type. Results Hyperprolactinemia (PRL-level: 16-24 μg/L) was found in 3.8% of the patients with RA. Prolactin levels were highest in premenopausal compared to postmenopausal females and males. Baseline PRL-levels were significantly lower in responders (median (range): 7.0 (2.0-24) μg/L) than in non-responders (9.3 (4.0-19) μg/L)) on TNF treatment (P=0.009). Higher tertiles of PRL (but within the physiological range) were associated with RF-positivity (P=0.005), aCCP-positivity (P=0.06) and erosive disease (P=0.024). After adjustment for these potential confounders, and for baseline-DAS28, baseline-PRL appeared to be a predictor of non-response to anti-TNF treatment (OR: 4.5; P=0.018; table 1). Table 1. Baseline PRL independently predicts a non-respons to anti-TNF treatment Exp (B)P Prolactin4.50.018 DAS280.260.000 Age0.940.018 Gender0.9560.949 RF and aCCP did not independently contribute. The proportion of patients expressing PRLR in the synovium was similar in RA (66%) and PsA (73%) patients, and lower in OA patients (25%; P=0.05). PRLR expression was higher in RA (median (range): 0.055 (0.000-5.673) IOD/nuclei/mm2) and PsA (0.182 (0.000-5.336)) compared to OA (0.000 (0.000-0.908); P=0.024). Males and (pre-/postmenopausal) females had similar PRLR expression. Using IF, co-localisation was observed with macrophages and endothelial cells. Conclusions Higher levels of PRL independently predicts a non-response to anti-TNF treatment. The expression of the PRLR in synovial tissue, mainly by macrophages, is higher in the inflammatory diseases (RA and PsA) than in OA. Our combined data suggest an important role of prolactin and its receptor in RA. Disclosure of Interest None Declared
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